von Willebrand's disease

von Willebrand's disease

 [von vil´ĕ-brahnts]

a congenital bleeding disorder, inherited as an autosomal dominant trait, characterized by a prolonged bleeding time, deficiency of von Willebrand's factor, and often impairment of platelet adhesion. It is associated with epistaxis and increased bleeding after trauma or surgery, menorrhagia, and postpartum bleeding. Called also angiohemophilia and pseudohemophilia.

Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

von Willebrand's disease

A rare group of hereditary bleeding disorders, similar to HAEMOPHILIA but usually less sevee, due to deficiency of a clotting factor necessary for PLATELET adhesion to blood vessel walls and as a carrier for Factor VIII. The condition is usually inherited as an autosomal dominant and, unlike haemophilia, occurs equally in both sexes. Some cases are automal recessive. The mutations, which are many, are on the short arm of chromosome 12. The von Willebrand factor (vWF) is a protein, the levels of which are raised in pregnancy. (E. A. von Willebrand, 1870–1949, Swedish physician).

Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005

von Willebrand's disease

An inherited lifelong bleeding disorder caused by an abnormal gene, similar to hemophilia. The gene defect results in a decreased blood concentration of a substance called von Willebrand's factor (vWF).

Mentioned in: Platelet Aggregation Test

Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.